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Multiple Coronary Artery-Left Ventricular Fistulas Associated With Hereditary Hemorrhagic Telangiectasia*
Coronary artery-left ventricular (LV) fistulas are extremely rare and can cause myocardial ischemia from coronary steal. We describe an elderly woman who presented with unstable angina from multiple and extensive coronary artery-LV fistulas. She also had clinical features suggestive of hereditary hemorrhagic telangiectasia (HHT). Association of coronary artery-LV fistulas with HHT has not been reported and can pose a management dilemma in view of the risks of extensive cardiopulmonary surgery and potential complications of myocardial ischemia, stroke, and brain abscess.
(CHEST 2001; 120:1415-1417)
Key words: adult; coronary artery fistula; coronary steal; hereditary hemorrhagic telangiectasia; Osler-Rendu-Weber syndrome; pulmonary arteriovenous fistula
Abbreviations: CAD = coronary artery disease; CAF = coronary artery fistula; HHT = hereditary hemorrhagic telangiectasia; LV = left ventricular
Coronary artery fistula (CAFs) are rare and are found in approximately 0.1% of patients undergoing cardiac catheterization.1 CAF involving all three major cardiac vessels and emptying into the left ventricle (arteriosystemic fistulas) are extremely uncommon. They are usually asymptomatic but can cause myocardial ischemia due to coronary steal mechanism, congestive heart failure, infecfive endocarditis, and rupture or thrombosis of the fistula.2-3 We present an elderly woman admitted to the hospital with unstable angina and subsequently found to have extensive coronary artery-left ventricular (LV) fistulas, pulmonary arteriovenous shunting, and mucocutaneous telangiectasia suggesting hereditary hemorrhagic telangiectasia (HHT; Osler-Rendu-Weber syndrome). To our knowledge, CAFs associated with HHT have not been reported.
CASE REPORT
A 72-year-old woman with chronic atrial fibrillation, hypertensive heart disease, and hypothyroidism presented with recurrent episodes of classical angina associated with palpitations. Family history was significant for premature coronary artery disease (CAD) and negative for pulmonary disease, cirrhosis, or bleeding diathesis. Her medications included atenolol, digoxin, warfarin, furosemide, conjugated estrogen, and L-thyroxine. A physical examination...